APSR RESPIRATORY UPDATES - apsresp.org · risk strata. The hazard ratio (HR) for death conferred by...
Transcript of APSR RESPIRATORY UPDATES - apsresp.org · risk strata. The hazard ratio (HR) for death conferred by...
![Page 1: APSR RESPIRATORY UPDATES - apsresp.org · risk strata. The hazard ratio (HR) for death conferred by a one point increase in the BACI was The hazard ratio (HR) for death conferred](https://reader030.fdocuments.in/reader030/viewer/2022041123/5d2280e288c99333128b7d24/html5/thumbnails/1.jpg)
To advertise, subscribe a colleague or to unsubscribe please contact : Secretariat, Asian Pacific Society of Respirology, 2F, UK's Bldg. 2-29-3 Hongo, Bunkyo-ku, Tokyo 113-0033 Japan. Email: [email protected]
Inside this issue: Bronchiectasis.
Adult Patients with Bronchiectasis. A first look at the US Bronchiectasis Research Registry. 2
Research priorities in bronchiectasis: a consensus statement from the EMBARC Clinical Research Col-laboration.
3
Aetiology of bronchiectasis in adults: A systematic literature review. 4
Multi-dimensional severity assessment in bronchiectasis: an analysis of seven European cohorts. 4
Comorbidities and the risk of mortality in patients with bronchiectasis: an international cohort study. 5
Bronchiectasis severity is an independent risk factor for vascular disease in a bronchiectasis cohort. 6
A randomised controlled trial of atorvastatin in patients with bronchiectasis infected with Pseudomo-nas aerigunosa. A proof of concept study.
7
Bronchoarterial ratio in non-smoking adults: implications for bronchial dilation definition. 8
Pseudomonas aeruginosa adaption and diversification in the non-cystic fibrosis bronchiectasis lung. 9
FUT2 Genotype Influences lung function, exacerbation frequency and airway microbiota in non-CF bronchiectasis.
10
The development and validation of the Bronchiectasis Health Questionnaire (BHQ). 11
Pulmonary exacerbation in adults with bronchiectasis: a consensus definition for clinical research. 11
The BRICS (Bronchiectasis Radiologically Indexed CT Score). A multicentre study score for use in idio-pathic and post-infective bronchiectasis.
12
APSR EDUCATION PUBLICATION
Newsletter Date: July 2018 Volume 10 Issue 7
APSR RESPIRATORY UPDATES
Articles selected and commented on by: Professor John Kolbe. Department of Medicine, Faculty of Medi-cal and Health Sciences, University of Auckland, Auckland City Hospital, Auckland, New Zealand
Keep informed about the latest news and
published articles in Respirology and
Respirology Case Reports with direct links to the articles! Sign up to follow us on
Twitter today!
![Page 2: APSR RESPIRATORY UPDATES - apsresp.org · risk strata. The hazard ratio (HR) for death conferred by a one point increase in the BACI was The hazard ratio (HR) for death conferred](https://reader030.fdocuments.in/reader030/viewer/2022041123/5d2280e288c99333128b7d24/html5/thumbnails/2.jpg)
To advertise, subscribe a colleague or to unsubscribe please contact : Secretariat, Asian Pacific Society of Respirology, 2F, UK's Bldg. 2-29-3 Hongo, Bunkyo-ku, Tokyo 113-0033 Japan. Email: [email protected]
Page 2 APSR RESPIRATORY UPDATES
That this is the second APSR Respiratory Update solely on the topic of bronchiectasis, reflects
the fact that this condition is, in the words of Professor Paul Torzillo, “shaking off its orphan
status” (Lancet Respiratory Medicine 2016; 4:927-928). This enhanced status is further re-
flected in the continued expansion of publications on the condition providing an increasingly
secure base for management, and in the early publications based on databases established
in the US and Europe. Pleasingly the name “bronchiectasis” has been reclaimed and the the
term “non-CF bronchiectasis” is being used much less frequently.
INTRODUCTION by Professor John Kolbe.
Authors: Aksomit T R et al
Reference: Chest 2017; 151: 982-992
URL:https://doi.org/10.1016/j.chest.2016.10.055
This is the first report arising from the US Bronchiectasis Research Registry, based on 13
participating centres and the enrolment of 1826 patients between 2008 and 2014. Patients
were predominantly elderly (mean age of 64 years), white (79%), female (79%), non smok-
ers (60%) with non-tuberculous mycobacterial infection (NTM) (63%) – with mostly MAIC
but about 20% were M. abcessus/chelonae. These are interesting demographic data which
seemingly fit the profile of those with the so-called “Lady Windermere’s Syndrome”. Howev-
er they may not be representative of the overall bronchiectasis population and it was
acknowledged that the cohort was enrolled from tertiary institutions with an interest in NTM.
The right middle lobe was the most frequently involved lobe (69%) but the more widespread
nature of the disease was reflected in the fact that only 11% had single lobe involvement.
Despite being the mainstay of management with proven benefit in terms of quality of life,
sputum volume and exercise capacity, only 56% were using “non-pharmacologic measures
to improve bronchial hygiene” with most using a Flutter device or PEP valve.
Another publication entitled “ Pharmacotherapy for non -cystic fibrosis bronchiecta-
sis” (Chest 2017; 152(6): 1120 – 1127; https://doi.org/10.1016/j.chest.2017.04.167) analysed
data from this registry (but now entitled “Bronchiectasis and NTM Research Registry”) and
from survey responders from a non-profit NTM patient advocacy organisation. The results
are noteworthy for the high rate of use of inhaled corticosteroids – 36% in those with NTM
and 42% in those without. This is despite the Cochrane Systematic Review on the subject
concluding that there is no convincing evidence of benefit from the use of inhaled cortico-
Adult Patients with Bronchiectasis. A first look at the US Bronchiectasis Research Registry.
![Page 3: APSR RESPIRATORY UPDATES - apsresp.org · risk strata. The hazard ratio (HR) for death conferred by a one point increase in the BACI was The hazard ratio (HR) for death conferred](https://reader030.fdocuments.in/reader030/viewer/2022041123/5d2280e288c99333128b7d24/html5/thumbnails/3.jpg)
To advertise, subscribe a colleague or to unsubscribe please contact : Secretariat, Asian Pacific Society of Respirology, 2F, UK's Bldg. 2-29-3 Hongo, Bunkyo-ku, Tokyo 113-0033 Japan. Email: [email protected]
Page 3 APSR RESPIRATORY UPDATES
steroids (ICS) in this condition, and that in chronic airways disease the use of ICS seem-
ingly increases the risk of NTM infection.
While the establishment of the US registry is a tremendous step forward, highlighting the
condition and advocating on behalf of patients, the inclusion of such a selective population
has the potential to distort our understanding of this condition. The challenge is now to ex-
pand the registry, to recruit from a wider range of centres, to be more geographically repre-
sentative, and to include ethnic minorities – and thus be more representative of the overall
bronchiectasis population and produce data that is more generalizable.
Author: Aliberti et al
Reference: European Respiratory Journal 2016; 48: 632 – 647
URL: http://erj.ersjournals.com/content/48/3/632.long
Further demonstrating the renewed interest in bronchiectasis, the European Multicentre
Bronchiectasis Audit and Research Collaboration (EMBARC) has been established under
the auspices of the European Respiratory Society, “to promote high quality research in
bronchiectasis”. This publication outlines the 55(!) key research priorities in this area, es-
tablished by consensus by European bronchiectasis experts, patients and their families and
resulting in 22 summary recommendations. These recommendations range from the estab-
lishment of DNA biobanks to enable study of genetic susceptibility, through observational
studies of large cohorts to better understand the natural history of the condition, to a num-
ber of randomised controlled trials of management strategies. This exercise to define key
research priorities is an ambitious undertaking that will serve to focus much needed re-
search in forthcoming years. One of the strengths of this endeavour is that it brought to-
gether the perspectives of “experts” and patients to produce a shared vision of research pri-
orities.
The European Respiratory Society has also published “Guidelines for the Management of
Adult Bronchiectasis”. (European Respiratory Journal 2017; 50:1-22). The format of this
document, developed by a multidisciplinary group, is to comprehensively address nine, clini-
cally highly relevant questions, providing recommendations, evidence, justification and com-
mentary on each of the specific issues. As well as being highly informative, this article pro-
vides management recommendations that could be used for audit, benchmarking and quali-
ty improvement. However, relevant to the EMBARC research priorities, was the acknowl-
edged low quality of evidence for a number of the recommendations.
Research priorities in bronchiectasis: a consensus statement from the EMBARC Clinical Research Collaboration.
![Page 4: APSR RESPIRATORY UPDATES - apsresp.org · risk strata. The hazard ratio (HR) for death conferred by a one point increase in the BACI was The hazard ratio (HR) for death conferred](https://reader030.fdocuments.in/reader030/viewer/2022041123/5d2280e288c99333128b7d24/html5/thumbnails/4.jpg)
To advertise, subscribe a colleague or to unsubscribe please contact : Secretariat, Asian Pacific Society of Respirology, 2F, UK's Bldg. 2-29-3 Hongo, Bunkyo-ku, Tokyo 113-0033 Japan. Email: [email protected]
Page 4 APSR RESPIRATORY UPDATES
Author: Gao Y-H, et al
Reference: Respirology 2016; 21: 1376-1383
URL: https://onlinelibrary.wiley.com/doi/full/10.1111/resp.12832
This systematic literature review identified 56 studies of a total of 8608 adults with bronchi-
ectasis. Reflecting the lack of a consistent definition for “idiopathic bronchiectasis” but also
possibly regional differences and referral bias, the crude prevalence for identified aetiolo-
gies ranged from 18 to 95%. Overall an underlying cause could not be identified in about
half (44.8%). Of the “known” aetiologies, post infection was the most frequent (29.9%), of
which post-tuberculosis was most common. Identification of an etiology that led to a change
in management occurred in only 18%.
However there are recognised specific aetiologies/associations of bronchiectasis and some
of these have recently been reviewed; bronchiectasis-rheumatoid overlap syndrome ( Chest
2017; 151(6): 1247 – 54), the yellow-nail syndrome (Respirology 2017; 22: 10177) and pri-
mary ciliary dyskinesia (Expert Review of Respiratory Medicine 2017; 11: 779 – 90).
Aetiology of bronchiectasis in adults: A systematic literature review.
Multi-dimensional severity assessment in bronchiectasis: an analysis of
seven European cohorts.
Authors: McDonnell MJ et al
Reference: Thorax 2016:71:1110-1118
URL: https://www.ncbi.nlm.nih.gov/pmc/articles/pmid/27516225/
In a previous APSR Respiratory Update, the two composite, disease-specific, clinical predic-
tion tools (the Bronchiectasis Severity Index (BSI) and FACED score) were reviewed. A re-
cently published single centre cohort analysis extended these findings by demonstrating that
both scores were able to predict 15-year mortality, but with the FACED score showing slightly
superior predictive power (Eur Respir J 2016; 47: 482-9).
This paper describes the head-to-head comparison of the predictive utility of these two instru-
ments in a large, combined European cohort (n=1612). As there is a considerable commonali-
ty in the factors included in these indices, it is not surprising that both scores had a good dis-
criminatory predictive value for mortality. However the BSI demonstrated a higher sensitivity
(65% vs 28%) but lower specificity (70 vs 93%) compared with the FACED score. The BSI
![Page 5: APSR RESPIRATORY UPDATES - apsresp.org · risk strata. The hazard ratio (HR) for death conferred by a one point increase in the BACI was The hazard ratio (HR) for death conferred](https://reader030.fdocuments.in/reader030/viewer/2022041123/5d2280e288c99333128b7d24/html5/thumbnails/5.jpg)
To advertise, subscribe a colleague or to unsubscribe please contact : Secretariat, Asian Pacific Society of Respirology, 2F, UK's Bldg. 2-29-3 Hongo, Bunkyo-ku, Tokyo 113-0033 Japan. Email: [email protected]
Page 5 APSR RESPIRATORY UPDATES
Comorbidities and the risk of mortality in patients with bronchiectasis: an
international cohort study.
Author: McDonnell MJ et al
Reference: Lancet Respiratory Medicine 2016; 4: 969 – 79
URL: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5369638/
The Bronchiectasis Severity Index (BSI) and the FACED score were both developed and vali-
dated in large cohorts, to predict mortality and morbidity in bronchiectasis. Understandably
these indices focussed on clinical, physiological, radiologic and microbiological features of bron-
chiectasis. However, bronchiectasis patients frequently have comorbidities that may be
“causative, synergistic or coincidental ….”. These comorbidities may contribute to symptoms,
functional impairment, morbidity, including hospitalisations, and mortality in patients with bron-
chiectasis.
In an observational cohort analysis of 986 bronchiectasis patients in four European centres,
comorbidities were defined and used to develop the Bronchiectasis Aetiology Comorbidity Index
(BACI). The median number of comorbidities per patient was four. Thirteen comorbidities
which independently predicted mortality were integrated into the BACI. The BACI predicted five
year mortality, hospitalisations, exacerbations and health-related quality of life, across all BSI
risk strata. The hazard ratio (HR) for death conferred by a one point increase in the BACI was
1. 18 (95%, 1.14 – 1.23); individual comorbidities attracted a score which ranged from 12
(metastatic malignancy) to 2 (peripheral vascular disease and ischaemic heart disease). COPD,
inflammatory bowel disease, connective tissue disease and asthma were also associated with a
performed more consistently across all degrees of severity and was superior to FACED in pre-
dicting other clinically useful outcomes including hospital admissions, exacerbations, quality of
life, respiratory symptoms, exercise capacity and lung function decline. This is not surprising
as the FACED was developed to specifically predict mortality while the BSI was developed to
predict mortality and other outcomes. While the BSI may be more useful to stratify patients on
the basis of risk of future exacerbations, it needs to be borne in mind that the BSI is more
complex than FACED.
In practical terms, the BSI may enable the identification of “high risk” patients in terms of cur-
rent symptom burden or likely future morbidity for whom more intensive treatment would be
indicated, and “low risk” patients who may require less intensive therapy and non-specialist
follow-up.
These instruments have only been evaluated in European cohorts and studies validating
these instruments in the Asia-Pacific populations would seem to be required.
![Page 6: APSR RESPIRATORY UPDATES - apsresp.org · risk strata. The hazard ratio (HR) for death conferred by a one point increase in the BACI was The hazard ratio (HR) for death conferred](https://reader030.fdocuments.in/reader030/viewer/2022041123/5d2280e288c99333128b7d24/html5/thumbnails/6.jpg)
To advertise, subscribe a colleague or to unsubscribe please contact : Secretariat, Asian Pacific Society of Respirology, 2F, UK's Bldg. 2-29-3 Hongo, Bunkyo-ku, Tokyo 113-0033 Japan. Email: [email protected]
Page 6 APSR RESPIRATORY UPDATES
higher risk of mortality. Even when a model was developed excluding conditions considered to
be aetiologically significant, the HR was very similar (1.17). The BACI was validated in an in-
dependent cohort of 113 patients. However a prediction model incorporating both BSI and
BACI was superior to either model alone for predicting five year mortality.
In summary, comorbidities are common in bronchiectasis, may be independent risk factors for
morbidity and mortality and may contribute to the systemic inflammation seen in this condition.
An online calculator for BACI is available at http://www.bronchiectasisseverity.com. At the very
least this publication reminds us of the importance of identifying, assessing and managing
comorbidities as part of the overall management of bronchiectasis.
Bronchiectasis severity is an independent risk factor for vascular disease in a bronchiectasis cohort.
Author: Evans IES et al
Reference: Chest 2017; 151: 383 – 8.
URL: https://doi.org/10.1016/j.chest.2016.09.022
Bronchiectasis is associated with systemic inflammation. A recent study, based on a retro-
spective database showed that bronchiectasis severity as determined by the BSI, was inde-
pendently associated with the development of vascular disease (ischaemic heart disease, cer-
ebrovascular disease, peripheral vascular disease and atrial fibrillation) after the diagnosis of
bronchiectasis. Bronchiectasis severity as determined by the BSI was independently associat-
ed with the development of vascular disease.
Further, a population-based study of almost four million persons demonstrated that pre-
existing diagnoses of coronary heart disease (CHD) and stroke were higher in persons with
bronchiectasis than those without bronchiectasis, after adjustment for other risk factors includ-
ing age, sex, smoking status and other risk factors for cardiovascular disease (Thorax 2017;
72:161 – 6). “In absolute terms our findings suggest that if a cohort of 100 people with bron-
chiectasis were followed up for five years, they would have three CHD events and five strokes
whereas 100 people without bronchiectasis would have one CHD event and one stroke.”
Thus there is the possibility that the pro-inflammatory state of bronchiectasis may have conse-
quences in terms of vascular diseases. This has relevance in terms of the influence of co-
morbidities on health care utilisation and mortality and on potential therapeutic interventions,
needs further consideration ( see next).
![Page 7: APSR RESPIRATORY UPDATES - apsresp.org · risk strata. The hazard ratio (HR) for death conferred by a one point increase in the BACI was The hazard ratio (HR) for death conferred](https://reader030.fdocuments.in/reader030/viewer/2022041123/5d2280e288c99333128b7d24/html5/thumbnails/7.jpg)
To advertise, subscribe a colleague or to unsubscribe please contact : Secretariat, Asian Pacific Society of Respirology, 2F, UK's Bldg. 2-29-3 Hongo, Bunkyo-ku, Tokyo 113-0033 Japan. Email: [email protected]
Page 7 APSR RESPIRATORY UPDATES
Author: Bedi P et al
Reference: Chest 2017; 152:368 – 78
URL: https://doi.org/10.1016/j.chest.2017.05.017
Bronchiectasis is associated with system inflammation and statin drugs have anti -
inflammatory (as well as other) properties. An earlier study by the same group from Edin-
burgh demonstrated that atorvastatin reduced cough, enhanced neutrophil apoptosis and
reduced CXCL8 (also called IL8) in patients with moderately severe bronchiectasis without
chronic infection with Pseudomonas aerigunosa (PA). (Lancet Respir Med 2014: 2: 455 -63.)
This double-blind, cross-over, randomised trial of 32 patients with more severe bronchiecta-
sis and chronic infection with PA, demonstrated that three month treatment with the statin
atorvastatin did not improve the primary outcome of cough (as assessed by Leicester
Cough Questionnaire) but did improve quality of life ( assessed by St George’s Respiratory
Questionnaire ) and reduced levels of a number of inflammatory cytokines including CXCL8,
tumour necrosis factor and intercellular adhesion molecule 1.
To determine whether statins have any role in the management of bronchiectasis will re-
quire a much longer study with important patient centred outcomes.
A randomised controlled trial of atorvastatin in patients with
bronchiectasis infected with Pseudomonas aerigunosa. A proof of concept study.
Coming soon in Respirology: “Paediatric and Adult Bronchiectasis” an invited review se-
ries co-edited by Adam Hill and Anne Chang.
Publication starts end of 2018.
Sign up on the Journal’s page to get content alert:
https://onlinelibrary.wiley.com/journal/14401843
![Page 8: APSR RESPIRATORY UPDATES - apsresp.org · risk strata. The hazard ratio (HR) for death conferred by a one point increase in the BACI was The hazard ratio (HR) for death conferred](https://reader030.fdocuments.in/reader030/viewer/2022041123/5d2280e288c99333128b7d24/html5/thumbnails/8.jpg)
To advertise, subscribe a colleague or to unsubscribe please contact : Secretariat, Asian Pacific Society of Respirology, 2F, UK's Bldg. 2-29-3 Hongo, Bunkyo-ku, Tokyo 113-0033 Japan. Email: [email protected]
Page 8 APSR RESPIRATORY UPDATES
Authors: Dias AA et al
Reference: Respirology 2017; 22: 108-113
URL: https://onlinelibrary.wiley.com/doi/full/10.1111/resp.12875
The defining radiologic feature of bronchiectasis is that of “airway dilation” based on the ra-
tio of the diameter of the bronchial lumen (B) and the adjacent pulmonary artery (A). A BA
ratio of >1 is the usual threshold for the radiologic diagnosis of bronchiectasis in adults.
The usual assumption is that an increased BA ratio is due to an increased airway lumen, but
could also be due to reduced artery calibre.
This study of the inspiratory CT scans of 106 never smokers, controls in the COPD Gene
study, mean age 62 years and 70% female, examined two bronchial paths; the right upper
lobe apical bronchus (RB1) and the right lower lobe posterior basal bronchus (RB10), cho-
sen because the airway branches of these segments are usually orthogonal to the axial
plane. The bronchial lumen and artery diameters were measured manually at points along
the path.
Overall the BA ratio was 0.79±0.16. In the RB1, but not the RB10 path, the BA ratio de-
creased in peripheral airway generations. The BA ratio was >1 in 8.5% of these normal
subjects. In those with BA>1, the artery diameter was smaller but bronchial lumen was not
larger than those with a BA ratio <1. In other words, the increased BA ratio is a broncho-
vascular process rather than solely a bronchial abnormality and in non-smokers a BA ratio
> 1 is driven by small arteries rather than luminal diameter. In multivariate analysis, BA
ratio was significantly associated with FEV1 and bronchial lumen size was more strongly
correlated with FEV1 than pulmonary artery diameter. These findings are consistent with
the concept of dysanapsis : that the bronchial tree and lung parenchyma may develop rela-
tively independently of each other. However these findings have important implications for
the diagnosis of bronchiectasis; clinically and epidemiologically.
All clinicians will be familiar with the unexpected radiologic reporting of mild thin walled
bronchiectasis, usually in the basal segments of the lower lobes in elderly patients who do
not have the clinical phenotype of bronchiectasis. Radiologic evidence of bronchiectasis
has been reported in up to a half of patients with severe COPD, and also in patients with
chronic severe asthma. In a study of the CT scans of 21 smokers with radiologic evidence
of bronchiectasis and 21 non-smoking controls, the BA ratios were higher in smokers with
mild bronchiectasis (as expected) but the increase was due to reduced calibre of the pulmo-
nary artery and not due to an increase in airway lumen size. Those with an increased BA
ratio had evidence of more severe airflow obstruction (Chest 2017; 151: 1255-62). Thus
Bronchoarterial ratio in non-smoking adults: implications for bronchial dilation definition.
![Page 9: APSR RESPIRATORY UPDATES - apsresp.org · risk strata. The hazard ratio (HR) for death conferred by a one point increase in the BACI was The hazard ratio (HR) for death conferred](https://reader030.fdocuments.in/reader030/viewer/2022041123/5d2280e288c99333128b7d24/html5/thumbnails/9.jpg)
To advertise, subscribe a colleague or to unsubscribe please contact : Secretariat, Asian Pacific Society of Respirology, 2F, UK's Bldg. 2-29-3 Hongo, Bunkyo-ku, Tokyo 113-0033 Japan. Email: [email protected]
Page 9 APSR RESPIRATORY UPDATES
smoking related increases in the BA ratio seem to be due to reductions in vascular calibre
rather than bronchial dilatation – termed “mistaken identity” in the accompanying editorial.
There would seem to be the need to review the use of artery diameter as a reference to de-
termine bronchial dilatation. The diagnosis of bronchiectasis is a clinical one and clinicians
need to interpret CT scans showing a BA ratio >1 in association with the patient’s clinical
features. Radiologic bronchiectasis may be common, but clinically significant bronchiectasis
may be much less common, in COPD .
Authors: Hilliam et al
Reference: European Respiratory Journal 2017; 49: 1602108.
URL: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5898933/
In cystic fibrosis, there is evidence of the coexistence of multiple divergent lineages of
Pseudomonas aerigunosa (PA), and a number of transmissible strains of PA have been
identified. Whether the same applies to bronchiectasis is unclear.
This study of whole genome sequencing of 189 PA isolates from 93 patients with bronchiec-
tasis from 16 centres in England and Wales demonstrated that the distribution of the PA iso-
lates from bronchiectasis patients were representative of the wider PA population. There
was evidence of multi-lineage infections, and some sharing of PA with the same clonal line-
age within the same clinic suggesting common source acquisition or cross-infection. A larg-
er longitudinal study will be needed to determine if there is person to person transmission of
PA in the bronchiectasis patient community.
As in CF, there was evidence of adaption of PA to the lung environment by the accumula-
tion of loss of function mutations, leading to changes in phenotype, including different
modes of iron acquisition, changes in twitching motility and variations in biofilm -associated
polysaccharides.
Pseudomonas aeruginosa adaption and diversification in the non-cystic fibrosis bronchiectasis lung.
APSR PUBLICATIONS
Edited By: Philip Bardin and Paul Reynolds
Impact Factor: 3.256
ISI Journal Citation Reports ©
Ranking:2016 18/59 (Respiratory System)
Online ISSN: 1440-1843
Edited By: Christopher Lai
Online ISSN: 2051-3380
![Page 10: APSR RESPIRATORY UPDATES - apsresp.org · risk strata. The hazard ratio (HR) for death conferred by a one point increase in the BACI was The hazard ratio (HR) for death conferred](https://reader030.fdocuments.in/reader030/viewer/2022041123/5d2280e288c99333128b7d24/html5/thumbnails/10.jpg)
To advertise, subscribe a colleague or to unsubscribe please contact : Secretariat, Asian Pacific Society of Respirology, 2F, UK's Bldg. 2-29-3 Hongo, Bunkyo-ku, Tokyo 113-0033 Japan. Email: [email protected]
Page 10 APSR RESPIRATORY UPDATES
Author: Taylor SC et al
Reference: Thorax 2017; 72: 304 – 310
URL: http://dx.doi.org/10.1136/thoraxjnl-2016-208775
Disease progression in bronchiectasis is variable and difficult to predict. One factor influ-
encing this progression is the airway microbiota; those with chronic Pseudomonas aeriguno-
sa (PA) infection having faster decline in lung function, more symptoms, worse quality of
life, more frequent pulmonary exacerbations and greater antibiotic requirements. Microbes
use glycans on the respiratory mucosal surface to adhere. Thus variability of the expres-
sion of these glycans may affect the susceptibility to infection by certain microbes and sub-
sequently the airway microbiome composition. The FUT2 gene encodes fucosyltranferases
which act to attach fucose to disaccharide precursors and hence control the expression of
ABO antigens on the epithelial mucin glycans. Homozygous loss of function mutations in the
FUT2 (secretor) gene results in the inability to express a number of glycans on the mucosal
surface.
Australian investigators studied the secretor genotype (the effect of loss of function muta-
tions of the FUT2 gene, both heterozygous and homozygous) on disease severity in 112
adults with bronchiectasis. FUT2 genotype was associated with disease progress; homozy-
gous secretors had lower lung function, greater number of exacerbations and a higher rate
of PA infection than homozygous non-secretors. Patients with a heterozygous genotype
demonstrated an intermediate phenotype. The authors speculated that the effect of secre-
tor status may be through susceptibility to respiratory viral infection. There is no direct link
between secretor status and PA infection.
Thus secretory genotype is a factor influencing type of chronic microbiological infection and
disease severity and progression in bronchiectasis. Bronchiectasis has a complex and in-
completely understood aetiology, but this may represent an early step towards the use of
precision medicine for a more effective approach to patient management in this condition.
FUT2 Genotype Influences lung function, exacerbation frequency and airway microbiota in non-CF bronchiectasis.
New in Respirology: Visual abstracts Visual abstract are gaining momentum in science publishing and prove valuable tools in the promotion and sharing of research findings online.
View our latest issue for featured visual abstracts.
![Page 11: APSR RESPIRATORY UPDATES - apsresp.org · risk strata. The hazard ratio (HR) for death conferred by a one point increase in the BACI was The hazard ratio (HR) for death conferred](https://reader030.fdocuments.in/reader030/viewer/2022041123/5d2280e288c99333128b7d24/html5/thumbnails/11.jpg)
To advertise, subscribe a colleague or to unsubscribe please contact : Secretariat, Asian Pacific Society of Respirology, 2F, UK's Bldg. 2-29-3 Hongo, Bunkyo-ku, Tokyo 113-0033 Japan. Email: [email protected]
Page 11 APSR RESPIRATORY UPDATES
Authors: Spinou A et al
Reference: European Respiratory Journal 2017; 49:
URL: http://erj.ersjournals.com/content/49/5/1601532.long
The authors of the editorial accompanying this article emphasise that there are three differ-
ent dimensions of any disease; severity, activity, and impact, and that the last of these is
usually evaluated by quality of life questionnaires. In a previous APSR Respiratory Update,
the report of the first disease -specific quality of life questionnaire for bronchiectasis (Quality
of Life – Bronchiectasis (QoL-B)) was reviewed.
This paper reports on the development and validation of another disease-specific quality of
life questionnaire – the Bronchiectasis Health Questionnaire (BHQ). This BHQ is simpler; it
has 10 items in one domain, compared with the 37 items and eight domains of the Qol-B.
The BHQ provides a single overall health status score which was shown to correlate with
numbers of exacerbations and hospitalisations, radiologic severity and bacterial colonisa-
tion. The Questionnaire has been translated into a number of languages including Japa-
nese and Mandarin and has been tested in a single center in China. However the minimally
clinically significant difference has not yet been determined, nor has the instrument’s re-
sponsiveness to interventions. While the BHQ meets the criteria for a good quality instru-
ment in terms of internal consistency, validity (most items reflect a good quality clinical his-
tory) and repeatability, it does not quantify health status in the psychological, activity and
social domains. Thus while the BHQ has the great advantage of brevity and simplicity, the
QoL-B has advantages if a more in-depth analysis of health status is required, particularly in
certain specific domains.
Authors: Hill AT et al
Reference: Eur Respir J 2017; 49: 1700051.
URL: http://erj.ersjournals.com/content/49/6/1700051.long
This consensus definition of bronchiectasis was “unanimously approved” by a multinational,
broad-based group of experts following a systematic review of the literature, a Delphi pro-
cess and a round table discussion. An exacerbation for the purposes of clinical research re-
quires at least 3 of 6 symptoms for 48 hrs and a clinician determining that a change in treat-
Pulmonary exacerbation in adults with bronchiectasis: a consensus definition for clinical research.
The development and validation of the Bronchiectasis Health Questionnaire (BHQ).
![Page 12: APSR RESPIRATORY UPDATES - apsresp.org · risk strata. The hazard ratio (HR) for death conferred by a one point increase in the BACI was The hazard ratio (HR) for death conferred](https://reader030.fdocuments.in/reader030/viewer/2022041123/5d2280e288c99333128b7d24/html5/thumbnails/12.jpg)
To advertise, subscribe a colleague or to unsubscribe please contact : Secretariat, Asian Pacific Society of Respirology, 2F, UK's Bldg. 2-29-3 Hongo, Bunkyo-ku, Tokyo 113-0033 Japan. Email: [email protected]
Page 12 APSR RESPIRATORY UPDATES
ment is indicated. Thus the definition includes 3 features - the development of symptoms,
duration of symptoms and a management change. While there are no surprises in the defi-
nition, the development of such an agreed definition of an oft-used primary outcome is an
important step forward for standardisation of clinical research in this condition.
Authors: Bedi P et al
Reference: Chest 2018; 153: 1177-1186
URL: https://doi.org/10.1016/j.chest.2017.11.033
This paper describes the development of a simplified radiologic score (BRICS) in 184 pa-
tients. Patients who were current smokers or had a >5 pack year smoking history were ex-
cluded. The score was based on a multivariable analysis of components of the Bhalla
score (Radiology 1991; 179: 783-788) and included those components that were significant-
ly associated with the disease severity markers viz degree of bronchial dilatation and num-
ber of broncho-pulmonary segments with emphysema. The score for BRICS ranged from 0
to 5; 1 indicated mild disease, 2 – 3 indicated moderate disease and 4 – 5 indicated severe
disease. The ROC curve values in the derivation cohort were 0.79 for per cent predicted
FEV1, 0.71 for sputum purulence and 0.75 for hospital admissions per year. Similar ROC
curve values were obtained in the validation cohort of 302 patients in six centres.
The higher levels of neutrophil elastase activity in the group with emphysema on CT scan
suggested that elastase may be the cause of emphysema in this group. There was a strong
correlation between the severity of the BRICS and the severity of the BSI and FACED score
(p<0.001 for both scores). However neither the BSI or FACED scores were significantly
correlated with percent predicted FEV1, sputum purulence or hospital admissions for bron-
chiectasis exacerbations.
The BRICS is a simple, robust scoring system based on CT features alone, that correlates
significantly with important clinical parameters and predicts disease severity in patients with
idiopathic and post-infective bronchiectasis.
The BRICS (Bronchiectasis Radiologically Indexed CT Score). A multicentre study score for use in idiopathic and post-infective
bronchiectasis.
![Page 13: APSR RESPIRATORY UPDATES - apsresp.org · risk strata. The hazard ratio (HR) for death conferred by a one point increase in the BACI was The hazard ratio (HR) for death conferred](https://reader030.fdocuments.in/reader030/viewer/2022041123/5d2280e288c99333128b7d24/html5/thumbnails/13.jpg)
To advertise, subscribe a colleague or to unsubscribe please contact : Secretariat, Asian Pacific Society of Respirology, 2F, UK's Bldg. 2-29-3 Hongo, Bunkyo-ku, Tokyo 113-0033 Japan. Email: [email protected]
Disclaimer: This publication is not intended as a replacement for regular medical education. The comments are an interpretation of the published study and reflect the opinion of the writer rather than those of the research group or scientific journal. It is suggested readers review the full trial data before forming a final conclusion on its merits. Privacy Policy: The APSR Secretariat will record your email details on a secure database and will not release it to anyone without your prior approval. The APSR and you have the right to inspect, update or delete your details at any time.
Asian Pacific Society of Respirology Page 13
APSR Respiratory Updates is an initiative of the APSR Education Committee
Articles selected and commented on by Professor John Kolbe, Department of Medicine, Faculty of Medical and Health Sciences, University of Auckland, Auckland City Hospital, Auckland, New Zealand
Editor in chief: Dr David CL Lam, Department of Medicine, University of Hong Kong, Hong Kong, China
Compiled by Dr Christel Norman, Respirology Editorial Office, Perth, Australia